melphalan and Cardiomyopathy--Restrictive

Amyloidosis is often difficult to diagnose and cardiac involvement worsens the prognosis.. We report the case of a 72-year old man consulting for cardiac failure with pleural effusion. A restrictive cardiomyopathy was discovered by echocardiography, and amyloidosis was then suspected. First histological localization was pleural. Cardiac involvement was confirmed. The diagnosis was supported by digestive and cutaneous localizations. It was an AL amyloidosis. Treatment with melphalan and dexamethasone allowed stabilization during more than six months.. This is an original case report, because of the first clinical signs (cardiac failure), the histological proof (pleural histology). Echocardiography is particularly helpful in internal medicine.

Topics: Aged; Amyloidosis; Anti-Inflammatory Agents; Antineoplastic Agents, Alkylating; Cardiomyopathy, Restrictive; Dexamethasone; Drug Therapy, Combination; Echocardiography; Humans; Male; Melphalan; Pleural Effusion; Treatment Outcome

Systemic light-chain deposition due to plasma cell dyscrasias manifests as a form of restrictive cardiomyopathy with diastolic ventricular dysfunction. Although these manifestations are likely to be cardiac amyloidosis, whether these pathological conditions are reversible after treatment of the underlying plasma cell disorders is unknown. To our knowledge, we describe the first patient with cardiac light-chain deposition due to multiple myeloma in whom echocardiographic and biochemical factors of cardiac function were ameliorated dramatically after remission of this disorder. We emphasize that restrictive cardiomyopathy due to light-chain deposition may be reversible and have a relatively better prognosis after remission of plasma cell dyscrasias.

Topics: Adult; Anorexia; Antineoplastic Combined Chemotherapy Protocols; Biopsy; Bone Marrow Examination; Cardiomyopathy, Restrictive; Dyspnea; Echocardiography; Electrocardiography; Hemodynamics; Humans; Immunoglobulin Light Chains; Male; Melphalan; Multiple Myeloma; Nitrosourea Compounds; Prognosis; Remission Induction; Treatment Outcome; Vincristine